中华儿科杂志
中華兒科雜誌
중화인과잡지
Chinese Journal of Pediatrics
2012年
12期
885-889
,共5页
王晓慧%方方%丁昌红%吕俊兰%韩彤立%刘丽英%李久伟%伍妘%崔丽英%任海涛%许春伶
王曉慧%方方%丁昌紅%呂俊蘭%韓彤立%劉麗英%李久偉%伍妘%崔麗英%任海濤%許春伶
왕효혜%방방%정창홍%려준란%한동립%류려영%리구위%오운%최려영%임해도%허춘령
受体,N-甲基-D-天冬氨酸%边缘叶脑炎%儿童
受體,N-甲基-D-天鼕氨痠%邊緣葉腦炎%兒童
수체,N-갑기-D-천동안산%변연협뇌염%인동
Receptors,N-methyl-D-aspartate%Limbic encephalitis%Child
目的 探讨儿童抗N-甲基-D-天冬氨酸(NMDA)受体脑炎临床特点和诊断,提高对该病的认识.方法 对确诊为抗NMDA受体脑炎患儿的临床表现、实验室及影像学资料进行回顾性分析.结果 本组7例,女4例,男3例,年龄6岁8个月至15岁6个月,平均年龄9岁6个月.4例以惊厥起病,6例病程中存在抗癫(癎)药物难以控制的惊厥发作；7例均有精神症状及语言障碍；6例具有不同程度意识障碍、锥体外系不自主运动,其中口舌面异常运动6例、舞蹈和(或)手足徐动5例、姿势异常5例；6例有自主神经功能障碍；7例存在睡眠障碍,多为失眠；5例存在进食困难；所有患儿头颅影像学检查大致正常；脑电图均不正常,多为局灶性或弥漫性慢波,3例有(癎)样放电；2例脑脊液细胞数增高,1例蛋白增高；6例脑脊液寡克隆区带检查阳性,未筛查出肿瘤.所有患儿均进行免疫治疗,4例在患病1～2个月左右开始恢复；1例在患病3个月开始好转.结论 儿童抗NMDA受体脑炎发病率比想象的高,并且该病在男童亦可见.临床表现与成人患者相似,但少见肿瘤,自主神经功能紊乱及中枢性低通气在儿童患者中发生率低,且程度轻.多数患儿预后良好.
目的 探討兒童抗N-甲基-D-天鼕氨痠(NMDA)受體腦炎臨床特點和診斷,提高對該病的認識.方法 對確診為抗NMDA受體腦炎患兒的臨床錶現、實驗室及影像學資料進行迴顧性分析.結果 本組7例,女4例,男3例,年齡6歲8箇月至15歲6箇月,平均年齡9歲6箇月.4例以驚厥起病,6例病程中存在抗癲(癎)藥物難以控製的驚厥髮作；7例均有精神癥狀及語言障礙；6例具有不同程度意識障礙、錐體外繫不自主運動,其中口舌麵異常運動6例、舞蹈和(或)手足徐動5例、姿勢異常5例；6例有自主神經功能障礙；7例存在睡眠障礙,多為失眠；5例存在進食睏難；所有患兒頭顱影像學檢查大緻正常；腦電圖均不正常,多為跼竈性或瀰漫性慢波,3例有(癎)樣放電；2例腦脊液細胞數增高,1例蛋白增高；6例腦脊液寡剋隆區帶檢查暘性,未篩查齣腫瘤.所有患兒均進行免疫治療,4例在患病1～2箇月左右開始恢複；1例在患病3箇月開始好轉.結論 兒童抗NMDA受體腦炎髮病率比想象的高,併且該病在男童亦可見.臨床錶現與成人患者相似,但少見腫瘤,自主神經功能紊亂及中樞性低通氣在兒童患者中髮生率低,且程度輕.多數患兒預後良好.
목적 탐토인동항N-갑기-D-천동안산(NMDA)수체뇌염림상특점화진단,제고대해병적인식.방법 대학진위항NMDA수체뇌염환인적림상표현、실험실급영상학자료진행회고성분석.결과 본조7례,녀4례,남3례,년령6세8개월지15세6개월,평균년령9세6개월.4례이량궐기병,6례병정중존재항전(간)약물난이공제적량궐발작；7례균유정신증상급어언장애；6례구유불동정도의식장애、추체외계불자주운동,기중구설면이상운동6례、무도화(혹)수족서동5례、자세이상5례；6례유자주신경공능장애；7례존재수면장애,다위실면；5례존재진식곤난；소유환인두로영상학검사대치정상；뇌전도균불정상,다위국조성혹미만성만파,3례유(간)양방전；2례뇌척액세포수증고,1례단백증고；6례뇌척액과극륭구대검사양성,미사사출종류.소유환인균진행면역치료,4례재환병1～2개월좌우개시회복；1례재환병3개월개시호전.결론 인동항NMDA수체뇌염발병솔비상상적고,병차해병재남동역가견.림상표현여성인환자상사,단소견종류,자주신경공능문란급중추성저통기재인동환자중발생솔저,차정도경.다수환인예후량호.
Objective To study the clinical and laboratory features and diagnosis of the patient with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children.Method The data of clinical feature,laboratory findings,and radiological manifestation were reviewed and analyzed.Result Of the 7 patients,4 were female and 3 were male.The age of onset was from 6.6 to 15.5 years (average 9.5 years).The onset of 4 cases started with convulsion.Six cases had seizures which was difficult to control by antiepileptic drugs.All patients had psychiatric symptoms and speech disorder.Six cases had different levels of decreased consciousness and dyskinesias.6 cases had autonomic nerve instability,and 7 cases developed sleep disorders.The results of MRI examination were normal in all patients.The EEG of most patients showed focal or diffuse slow waves.Six cases had oligoclonal bands.All cases were confirmed to have the disease by detection of anti-NMDA receptor antibodies.No tumor was detected in any of the patients.All patients received immunotherapy.Conclusion Anti-NMDAR encephalitis is a severe but treatable disorder that frequently affects children and adolescents.Pediatric patients had clinical manifestations similar to those of adult patients.But children have a lower incidence of tumors and hypoventilation also occurs less frequently in children.Most of children had a good prognosis.
